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Branched Chain Keto Acid Dehydrogenase E1, beta Polypeptide (BCKDHB) ELISA Kits

Branched-chain keto acid dehydrogenase is a multienzyme complex associated with the inner membrane of mitochondria, and functions in the catabolism of branched-chain amino acids. Additionally we are shipping Branched Chain Keto Acid Dehydrogenase E1, beta Polypeptide Antibodies (25) and Branched Chain Keto Acid Dehydrogenase E1, beta Polypeptide Proteins (6) and many more products for this protein.

list all ELISA KIts Gene Name GeneID UniProt
Anti-Rat BCKDHB BCKDHB 29711 P35738
Anti-Human BCKDHB BCKDHB 594 P21953
Anti-Mouse BCKDHB BCKDHB 12040 Q6P3A8
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More ELISA Kits for Branched Chain Keto Acid Dehydrogenase E1, beta Polypeptide Interaction Partners

Pig (Porcine) Branched Chain Keto Acid Dehydrogenase E1, beta Polypeptide (BCKDHB) interaction partners

  1. analysis of NCF2 (show NCF2 ELISA Kits), BCKDHB and BCKDHA (show BCKDHA ELISA Kits) in pig

Human Branched Chain Keto Acid Dehydrogenase E1, beta Polypeptide (BCKDHB) interaction partners

  1. we describe the presenting symptoms and clinical course of a Chinese boy with intermittent Intermittent maple syrup urine disease from BCKDHB gene mutation

  2. Mutations in BCKDHB gene is associated with maple syrup urine disease.

  3. BCKDHA (show BCKDHA ELISA Kits) and BCKDHB mutations might be primarily responsible for maple syrup urine disease in the Indian population.

  4. A key tyrosine residue in the E1b active site functions as a conformational switch to reduce the reactivity of the thiamine diphosphate cofactor through interactions with its thiazolium ring.

  5. the two active sites in the E1b heterotetramer operate independently during the ThDP-dependent decarboxylation reaction

  6. Reconstructed haplotype from BCKDHB variants was associated with premature ovarian failure.

  7. in our cohort more severe enzyme & clinical phenotypes of variant maple syrup urine disease were mainly associated with specific genotypes in BCKDHA (show BCKDHA ELISA Kits) gene; milder enzyme & clinical phenotypes were associated with specific genotypes in BCKDHB & DBT (show DBT ELISA Kits) genes

  8. 30 Maple syrup urine disease Portuguese patients studied; 17 putative mutations have been identified (6 in BCKDHA (show BCKDHA ELISA Kits), 5 in BCKDHB and 6 in DBT (show DBT ELISA Kits)); 7 of are described for the first time.

  9. In 37% (12 patients) of a total of 64 alleles, the supposed maple syrup urine disease-causing mutations in Turkish patients were located in the BCKDHA (show BCKDHA ELISA Kits) gene, in 44% (14 patients) in the BCKDHB gene and in 19% (6 patients) in the DBT (show DBT ELISA Kits) gene.

Mouse (Murine) Branched Chain Keto Acid Dehydrogenase E1, beta Polypeptide (BCKDHB) interaction partners

  1. Disruption of the BDK (show KNG1 ELISA Kits) gene establishes that regulation of BCKDH by phosphorylation is critically important for the regulation of oxidative disposal of branched-chain amino acids.

Branched Chain Keto Acid Dehydrogenase E1, beta Polypeptide (BCKDHB) Antigen Profile

Antigen Summary

Branched-chain keto acid dehydrogenase is a multienzyme complex associated with the inner membrane of mitochondria, and functions in the catabolism of branched-chain amino acids. The complex consists of multiple copies of 3 components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). This gene encodes the E1 beta subunit, and mutations therein have been associated with maple syrup urine disease (MSUD), type 1B, a disease characterized by a maple syrup odor to the urine in addition to mental and physical retardation, and feeding problems. Alternative splicing at this locus results in transcript variants with different 3' non-coding regions, but encoding the same isoform.

Gene names and symbols associated with BCKDHB

  • branched chain keto acid dehydrogenase E1, beta polypeptide (BCKDHB) antibody
  • branched chain ketoacid dehydrogenase E1, beta polypeptide (bckdhb) antibody
  • branched chain keto acid dehydrogenase E1, beta polypeptide (bckdhb) antibody
  • branched chain keto acid dehydrogenase E1, beta polypeptide (Bckdhb) antibody
  • branched chain ketoacid dehydrogenase E1, beta polypeptide (Bckdhb) antibody
  • BCKDHB antibody
  • dJ279A18.1 antibody
  • E1B antibody
  • zgc:158777 antibody

Protein level used designations for BCKDHB

2-oxoisovalerate dehydrogenase subunit beta, mitochondrial , branched chain keto acid dehydrogenase E1, beta polypeptide (maple syrup urine disease) , mitochondrial branched-chain alpha-keto acid dehydrogenase E1-beta subunit , branched chain keto acid dehydrogenase E1, beta polypeptide , branched chain keto acid dehydrogenase E1 beta polypeptide , branched chain keto acid dehydrogenase E1 beta-like , 2-oxoisovalerate dehydrogenase beta subunit , BCKDE1B , BCKDH E1-beta , branched chain keto acid dehydrogenase E1 beta , branched-chain alpha-keto acid dehydrogenase E1 component beta chain , E1b-beta subunit of the branched-chain complex , branched chain alpha-ketoacid dehydrogenase E1-beta subunit

GENE ID SPECIES
395375 Gallus gallus
462845 Pan troglodytes
474978 Canis lupus familiaris
569980 Danio rerio
100023799 Monodelphis domestica
100065300 Equus caballus
100137712 Xenopus laevis
100142669 Sus scrofa
100173401 Pongo abelii
100353682 Oryctolagus cuniculus
100597493 Nomascus leucogenys
29711 Rattus norvegicus
594 Homo sapiens
282150 Bos taurus
12040 Mus musculus
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